Juvenile ocular myasthenia gravis: presentation and outcome of a large cohort

Publication date: Available online 30 June 2018
Source:Pediatric Neurology
Author(s): Kavin Vanikieti, Kasamon Lowwongngam, Tanyatuth Padungkiatsagul, Anannit Visudtibhan, Anuchit Poonyathalang
BackgroundIsolated ocular myasthenia gravis (MG) is very common in children relative to adults, ranging from 71–93% of all children with MG.PurposeTo characterize the ocular manifestations and outcomes in children with isolated ocular MG.MethodsMedical records of consecutive 62 subjects, <15 years of age with ocular MG, were retrospectively reviewed. Demographic data, presenting ocular features, types and variabilities of duction limitation, MG confirmatory tests, types of and responses to treatment, and generalized MG conversion were reviewed.ResultsMean age at onset and follow-up time were 49 months (range, 1–173 months) and 95 months (range, 6–226 months), respectively. Female-to-male ratio was 1.5:1. Initially, ptosis was found in 60 subjects (96.8%), while duction limitation was observed in 28 subjects (45.2%). Total ophthalmoparesis was the most common type of duction limitation. Variability of duction limitation was found in 68% of subjects during the follow-up. Pyridostigmine alone was the most common medication used (48.4%); ptosis was more responsive than duction limitation. Generalized MG conversion was found in 19.4% of subjects, with a mean interval of 9 months after symptom onset. Most conversions (91.7%) occurred in the first two years.ConclusionsPtosis was more responsive than duction limitation significantly. Thus, other treatment modalities, as well as strabismic amblyopia screening, should be considered in children with prolonged duction limitation refractory to medication. In contrast with adults, a much lower proportion of children converted to generalized MG. This may explain the higher prevalence of isolated ocular MG among the juvenile population.



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